title: Characterization of the inflammatory cells in ascending thoracic aortic aneurysms in patients with Marfan syndrome, familial thoracic aortic aneurysms, and sporadic aneurysms.
abstract: OBJECTIVE
  This study sought to characterize the inflammatory infiltrate in ascending thoracic aortic aneurysm in patients with Marfan syndrome, familial thoracic aortic aneurysm, or nonfamilial thoracic aortic aneurysm.
  
  
  BACKGROUND
  Thoracic aortic aneurysms are associated with a pathologic lesion termed "medial degeneration," which is described as a noninflammatory lesion. Thoracic aortic aneurysms are a complication of Marfan syndrome and can be inherited in an autosomal dominant manner of familial thoracic aortic aneurysm.
  
  
  METHODS
  Full aortic segments were collected from patients undergoing elective repair with Marfan syndrome (n = 5), familial thoracic aortic aneurysm (n = 6), and thoracic aortic aneurysms (n = 9), along with control aortas (n = 5). Immunohistochemistry staining was performed using antibodies directed against markers of lymphocytes and macrophages. Real-time polymerase chain reaction analysis was performed to quantify the expression level of the T-cell receptor beta-chain variable region gene.
  
  
  RESULTS
  Immunohistochemistry of thoracic aortic aneurysm aortas demonstrated that the media and adventitia from Marfan syndrome, familial thoracic aortic aneurysm, and sporadic cases had increased numbers of T lymphocytes and macrophages when compared with control aortas. The number of T cells and macrophages in the aortic media of the aneurysm correlated inversely with the patient's age at the time of prophylactic surgical repair of the aorta. T-cell receptor profiling indicated a similar clonal nature of the T cells in the aortic wall in a majority of aneurysms, whether the patient had Marfan syndrome, familial thoracic aortic aneurysm, or sporadic disease.
  
  
  CONCLUSIONS
  These results indicate that the infiltration of inflammatory cells contributes to the pathogenesis of thoracic aortic aneurysms. Superantigen-driven stimulation of T lymphocytes in the aortic tissues of patients with thoracic aortic aneurysms may contribute to the initial immune response.
authors.affiliation: Department of Internal Medicine, The University of Texas Medical School at Houston, Houston, Texas 77030, USA.
authors.forename: Rumin
authors.initials: R
authors.lastname: He
authors.forename: Dong-Chuan
authors.initials: DC
authors.lastname: Guo
authors.forename: Wei
authors.initials: W
authors.lastname: Sun
authors.forename: Christina L
authors.initials: CL
authors.lastname: Papke
authors.forename: Senthil
authors.initials: S
authors.lastname: Duraisamy
authors.forename: Anthony L
authors.initials: AL
authors.lastname: Estrera
authors.forename: Hazim J
authors.initials: HJ
authors.lastname: Safi
authors.forename: Chul
authors.initials: C
authors.lastname: Ahn
authors.forename: L Maximilian
authors.initials: LM
authors.lastname: Buja
authors.forename: Frank C
authors.initials: FC
authors.lastname: Arnett
authors.forename: Jingwu
authors.initials: J
authors.lastname: Zhang
authors.forename: Yong-Jian
authors.initials: YJ
authors.lastname: Geng
authors.forename: Dianna M
authors.initials: DM
authors.lastname: Milewicz
mesh_terms: D000328:Adult; D000368:Aged; D015703:Antigens, CD / Q000276:immunology / Q000378:metabolism; D000945:Antigens, Differentiation, T-Lymphocyte / Q000235:genetics / Q000378:metabolism; D017544:Aortic Aneurysm, Abdominal / Q000276:immunology / Q000473:pathology* / Q000601:surgery; D017545:Aortic Aneurysm, Thoracic / Q000276:immunology / Q000473:pathology* / Q000601:surgery; D015415:Biomarkers / Q000032:analysis; D001707:Biopsy, Needle; D006348:Cardiac Surgical Procedures / Q000379:methods; D016022:Case-Control Studies; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008382:Marfan Syndrome / Q000235:genetics / Q000473:pathology* / Q000601:surgery; D008875:Middle Aged; D015999:Multivariate Analysis; D012313:RNA / Q000032:analysis; D011948:Receptors, Antigen, T-Cell / Q000276:immunology* / Q000378:metabolism; D012016:Reference Values; D020133:Reverse Transcriptase Polymerase Chain Reaction; D012680:Sensitivity and Specificity; D018709:Statistics, Nonparametric; D046509:Tissue Culture Techniques; D053781:Transforming Growth Factor beta2 / Q000032:analysis / Q000276:immunology*; D019010:Vascular Cell Adhesion Molecule-1 / Q000276:immunology / Q000378:metabolism
chemical_list: D015703:Antigens, CD; D000945:Antigens, Differentiation, T-Lymphocyte; D015415:Biomarkers; D011948:Receptors, Antigen, T-Cell; D053781:Transforming Growth Factor beta2; D019010:Vascular Cell Adhesion Molecule-1; D012313:RNA